Thalassemia

Clinical Features:

• 1)Recurrent infection 
• 2)Weakness.
• 3)Pale or yellowish skin.
• 4)Facial bone deformities.
• 5)Slow growth.
• 6)Abdominal swelling.
• 7)Dark urine.

On examination: 

• Anaemia:+++
  
• Jaundice:++
• Facial change : thalassemic facies(frontal& parital bossing,depressed nasal bridge,prominent zygoma& malalingned jaw & teeth.)
• Hepatosplenomegaly 

Investigations: 

• 1)CBC with PBF 
  
• 2)Serum iron & ferritin : increased 
• 3)serum transferin saturation : increased 
• 4)TIBC: decreased 
• 5)serum unconjugated bilirubin : increased 
• 6) Hb electrophoresis : ([post id="71" title="Hb-Electrophoresis"])(confirmatory) reduced or absent HbA, increase Hb F & HbA2[1st time Thalassemia confirm করার জন্য জন্য blood দেবার আগেই PBF & Hb electrophoresis দিতে হবে। blood দিলে picture change হয়ে যায় তাই সঠিক result আসে না 
• 7) X-ray skull - hair on end appearance 

Rx: 

• Counselling 
  
• Blood transfusion - PRBC, washed RBC,frozen RBC 
  

1.Tab.Folison 5mg 

0+1+0........3 month 

If serum ferritin level more then 1000ng/ml then start iron chelation agent 

দুটি transfusion মাঝখানে পাবে 

Cap.Kelfer(Deferiprone) 250mg 

• 1+1+1.....3 month 

OR

Tab.Asunra (Deferasirox)100mg

• 1+0+0.......3 month